Blistering disorders

Question 1

Autoimmune condition - antibodies against hemi desmosomes at the DEJ?

Answer 1

Bullous pemphigoid

Question 2

In bullous pemphigoid - interruption of what?

Results in?

Answer 2

DEJ

Sub epidermal blisters

Question 3

typical age of patient in bullous pemhigoid?

Answer 3

elderly

Question 4

what can present about 1 year prior to blisters in pemphigoid?

Answer 4

well demarcated erythematous plaques

Question 5

Typical blisters in pemphigoid ? size/consistency?

Answer 5

Large, tense itchy blisters on either normal or erythematous skin
no mucosal involvement

Question 6

Blisters in bullous pemphigoid (large and tense and itchy) typically found where on body?

Answer 6

trunk and proximal limbs

Question 7

Bullous pemphigoid - nikolsky sign negative or positive ?

Answer 7

negative (blisters dont rupture on rubbing skin)

Question 8

Dx of bullous pemphigoid ?

Answer 8

Biopsy and immunofluorescence

Question 9

On biopsy - sub epidermal blisters + inflammatory infiltrates within blister - Dx ?

Answer 9

Bullous pemphigoid

Question 10

Findings on immunofluorescence in bullous pemphigoid?

Answer 10

Linear IgG + complement deposition along the basement membrane

Question 11

Management of bullous pemphigoid:

a) local disease ?
b) systemic disease ?

Answer 11

a) topical steroids (potent)
b) oral steroids (mainstay of treatment) tetracycline ABs
anti-histamines

Question 12

use of drugs in bullous pemphigoid:

1) anti histamines ?
2) tetracyclines?

Answer 12

1) sedating and anti pruritic

2) steroid sparing agents

Question 13

No response to steroids ABs etc in bullous pemphigoid next line treatment is ?

Answer 13

Immunosupression

Question 14

Autoimmune condition - antibodies to desmoglein 3 (cell to cell adhesion) in epidermis Dx?

Answer 14

Pemphigus vulgaris

Question 15

Intra epidermal blisters which are weak and easily burst (nikolsky sign positive) Dx?

Answer 15

Pemphigus vulgaris

Question 16

Typical aged pt with pemphigus vulgaris ?

Answer 16

middle age

Question 17

Areas of skin affected in pemphigus vulgaris ?

Answer 17

scalp face axilla trunk
mucous membranes (mouth throat, rectum, conjunctivae)

Question 18

Typical rash in pemhigus vulgaris ?

Answer 18

multiple painful flaccid fragile blisters and skin erosions

Question 19

Dx Ix for pemhigus vulgaris?

Answer 19

Biopsy and immunofluroscence

Question 20

Biopsy in pemphigus vulgaris ?

Answer 20

Intra epidermal blister + accumulation of inflammatory cells within dermis

Question 21

Chicken wire deposition of IgG within the epidermis on immunofluorescence - Dx?

Answer 21

Pemphigus vulgaris

Question 22

Treatment of pemphigus vulgaris:

a) Local disease ?
b) systemic disease?

Answer 22

a) Topical steroids + topical anaesthetics

b) high dose oral steroids + immunosupression +/-rituximab

Question 23

Autoimmune skin condition - small intensly itchy blisters on an erythematous swollen base (symptoms of bowel symptoms) Dx?

Answer 23

Dermatitis herpetiformis

sub epidermal blisters

Question 24

autoantibodies against what in dermatitis herpetiformis ?

Answer 24

TTG tissue transglutaminase

Question 25

associated conditon in dermatitis herpetiformis ?

Answer 25

Coeliac disease

Question 26

With dermatitis herpetiformis (itch can precede blisters) - common location of lesions on body?

Answer 26

extensor aspects of elbows, forearms, knees, buttocks, scapulae, face and scalp

Question 27

Dx of dermatitis herpetiformis ?

Answer 27

Blood - anti TTG
Biopsy - sub epidermal blisters (micro abscesses)
Immunofluroescence

Question 28

IF findings in dermatitis herpetiformis?

Answer 28

Granular deposits of IgA within epidermis

Question 29

Treatment of dermatitis herpetiformis ?

with this conditon - small increased risk of what (rare)?

Answer 29

Gluten free diet + dapsone cream

small bowel lymphoma